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Titolo:
Sarcospan-deficient mice maintain normal muscle function
Autore:
Lebakken, CS; Venzke, DP; Hrstka, RF; Consolino, CM; Faulkner, JA; Williamson, RA; Campbell, KP;
Indirizzi:
Univ Iowa, Coll Med, Howard Hughes Med Inst, Dept Physiol, Iowa City, IA 52242 USA Univ Iowa Iowa City IA USA 52242 t, Dept Physiol, Iowa City, IA 52242 USA Univ Iowa, Coll Med, Howard Hughes Med Inst, Dept Neurol, Iowa City, IA 52242 USA Univ Iowa Iowa City IA USA 52242 st, Dept Neurol, Iowa City, IA 52242 USA Univ Iowa, Coll Med, Howard Hughes Med Inst, Dept Biophys, Iowa City, IA 52242 USA Univ Iowa Iowa City IA USA 52242 t, Dept Biophys, Iowa City, IA 52242 USA Univ Iowa, Coll Med, Dept Obstet & Gynecol, Iowa City, IA 52242 USA Univ Iowa Iowa City IA USA 52242 bstet & Gynecol, Iowa City, IA 52242 USA Univ Michigan, Inst Gerontol, Ann Arbor, MI 48109 USA Univ Michigan Ann Arbor MI USA 48109 st Gerontol, Ann Arbor, MI 48109 USA
Titolo Testata:
MOLECULAR AND CELLULAR BIOLOGY
fascicolo: 5, volume: 20, anno: 2000,
pagine: 1669 - 1677
SICI:
0270-7306(200003)20:5<1669:SMMNMF>2.0.ZU;2-P
Fonte:
ISI
Lingua:
ENG
Soggetto:
DYSTROPHIN-GLYCOPROTEIN COMPLEX; DUCHENNE MUSCULAR-DYSTROPHY; NITRIC-OXIDE SYNTHASE; JUNCTION INTERCELLULAR CHANNELS; FIBROBLAST GROWTH-FACTOR; SKELETAL-MUSCLE; GAMMA-SUBUNIT; SARCOGLYCAN COMPLEX; CALCIUM-CHANNEL; IN-VITRO;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Life Sciences
Citazioni:
73
Recensione:
Indirizzi per estratti:
Indirizzo: Campbell, KP Univ Iowa, Coll Med, Howard Hughes Med Inst, Dept Physiol, 400 Eckstein Med Res Bldg, Iowa City, IA 52242 USA Univ Iowa 400 Eckstein MedRes Bldg Iowa City IA USA 52242 SA
Citazione:
C.S. Lebakken et al., "Sarcospan-deficient mice maintain normal muscle function", MOL CELL B, 20(5), 2000, pp. 1669-1677

Abstract

Sarcospan is an integral membrane component of the dystrophin-glycoproteincomplex (DGC) found at the sarcolemma of striated and smooth muscle. The DGC plays important roles in muscle function and viability as evidenced by defects in components of the DGC, which cause muscular dystrophy. Sarcospan is unique among the components of the complex in that it contains four transmembrane domains with intracellular Nand C-terminal domains and is a member of the tetraspan superfamily of proteins. Sarcospan is tightly linked to the sarcoglycans, and together these proteins form a subcomplex within the DGC. Stable expression of sarcospan at the sarcolemma is dependent upon expression of the sarcoglycans. Here we describe the generation and analysis of mice carrying a null mutation in the Sspn gene. Surprisingly, the Sspn-deficient muscle maintains expression of other components of the DGC at the sarcolemma, and no gross histological abnormalities of muscle from the mice are observed. The Sspn-deficient muscle maintains sarcolemmal integrity as determined by serum creatine kinase and Evans blue uptake assays, and the Sspn-deficient muscle maintains normal force and power generation capabilities. These data suggest either that sarcospan is not required for normal DGCfunction or that the Sspn-deficient muscle is compensating for the absenceof sarcospan, perhaps by utilizing another protein to carry out its function.

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Documento generato il 04/12/20 alle ore 01:27:22